Opinion: Persian Tay-Sachs

Posted on Dec. 1, 2011 at 12:48 pm

In the story, a young prince in an old and distant kingdom is mesmerized with salt.

The prince loves the taste of salt on his tongue, the dryness of it on his skin. He loves the way the grains catch the light as they slip through his fingers like so many tiny pieces of glass on a sun-bleached day. While all the other children in the kingdom are doing what children do, the young prince indulges his fascination with salt. He’s so busy with his adoration, he forgets to grow and yet, even in adulthood, he cannot stop the craving for salt.

Disconsolate over his son’s plight, the king falls ill. In his white palace with emerald windows, his grand wizard, when called upon, ordains that to heal, the king must fall sleep and dream of beauty and youth and conquest. The king dreams he’s young again; he stands in a field of wild grass, watching a beautiful maiden with golden locks run toward him from the edge of the horizon. She’s a fast runner with a graceful step, so light on her feet, he wonders if they ever touch the ground, but the closer she comes to the king, the more slowly she runs. He watches as her gait becomes uneven and her legs fold and she falls to the ground. When it’s time for him to wake up, the king cannot bring himself to abandon the injured and struggling maiden in the grass. The grand wizard calls him; the queen and the prince beckon him; the king will not wake.

Years ago, in “Cry of the Peacock,” I wrote the story of my own family and of other Iranian Jews I had known or been told about. Most of what I wrote was real, but when the book was published the reviewers evoked terms like “fairy tale” and “magical realism.” I didn’t know what magical realism was, didn’t think I had written a fairy tale. If anyone asked, I tried to explain that what seemed “magical” to the Western ear was just a whole lot of “realism” in the East.

In the land of the slumbering monarch, the queen summons her husband’s army and enjoins them to search the land for a spell that will rouse him from sleep. The bravest of all these men, two brothers, go farther and look more assiduously than all the rest. Everywhere they stop, they tell the story of the boy who won’t grow and the king who won’t wake, and they even tell about the maiden in the king’s dream, how she could outrun the goddess Atlanta until she was betrayed by her own limbs, but no one can help. At the last hour of the last day of their quest, the two brothers encounter two wise men who have come to the kingdom from distant shores, and who claim to have the spell.

I wrote a second book about real events in real people’s lives, and I even wrote a third — this one about all the “magical” things that happen in the far and forgotten corners of these United States — and still, I couldn’t convince most people of the veracity of so much of what happens in the books. Nor did I have a logical explanation for the disconnect. All I knew was that, left alone and forgotten for 3,000 years before they immigrated to the West, Iranian Jews had seen the world become, like Alice’s Wonderland, “weirder and weirder.”

At the palace with the emerald windows, the two wise men draw the king from his sleep and free the prince of the curse of the sorcerer’s salt. And though they cannot save the girl with the faltering gait, they vow to deliver the kingdom of all its unusual maladies. The two wise men have rid other kingdoms of other strange and devastating curses, and they know they can do it again. All they ask for in exchange is a drop of saliva and a tin coin from each of the king’s subjects.

In the late 1990s, two young Iranian Jewish physicians announced the existence of an illness that may be unique to Iranian Jews. At first, hardly anyone believed them. Later, most people thought that even if such an illness did exist, it was so rare as to be irrelevant to the general population. In 2001, Hadassah University Hospital in Israel announced the discovery of a gene that, in its mutated form, can cause a progressive muscular disease that attacks the individual in his 20s; it begins with a weakening of the leg muscles and gradually spreads through the body. The illness is called hereditary inclusion body myopathy, or HIBM. One in 20 Iranian Jews is a carrier of the gene.

Other recessive genetic disorders in Iranian Jews were soon identified: a sensitivity to some forms of anesthesia that can result in death (one in 10 is a carrier); a salt-losing disorder that causes great craving for salt and, if untreated, short stature (one in 30 is a carrier); a hormone deficiency that can lead to, among other ailments, diabetes mellitus type 1 (one in 50 is a carrier).

Although gene mutations exist in every ethnic and racial group, they are more common, and therefore easier to find, in isolated societies with a history of intermarriage. Among Ashkenazi Jews, Tay-Sachs disease was the evil giant that remained latent in a person’s body until he or she produced a child with a partner who was also a carrier of the gene. Tay-Sachs was eliminated in the Ashkenazi population thanks to genetic testing and premarital counseling that were a result of the efforts of two of the world’s leading geneticists, Dr. David Rimoin and Dr. Michael Kaback.

The two wise men stand at the gates of the white palace ready to save the denizens of the old and distant realm. They wait, but no one comes. Bewildered, they send couriers and emissaries into the heartland and repeat their pledge. They sound the bells and call from the highest towers and still, no one answers.

Forty years after spearheading the Tay-Sachs project, Drs. Rimoin and Kaback undertook the task of eradicating Iranian Jewish genetic illnesses. At the Cedars-Sinai Medical Genetics Institute, under Dr. Rimoin’s leadership, Iranian Jewish couples who are planning a family can be tested to make sure they’re not both carriers of the gene; if they are, they can guard against having children who are affected by one illness or another by undergoing in-vitro fertilization and choosing not to implant the defective embryos. Parents with small children can detect some illnesses in time to treat them. And even older people can alert their physicians of their sensitivity to some anesthesia medicines. Testing is entirely confidential, and by law, insurance companies cannot deny coverage or raise rates based on test results. The test costs $350 and requires only a few drops of saliva.

And yet, in the two years since a thousand Iranian Jews were tested under the pilot program at Cedars-Sinai, the phones at the center have remained quiet and few requests have been made for testing. Many Iranian Jews have yet to learn about the diseases or the fact that the genes have been identified. Many fear that by speaking openly about the illnesses and the testing, the community would expose itself to adverse reactions from Ashkenazi Jews. Many do not have the $350 to spare. So far, the dozens of religious and cultural organizations, the nonprofits and all the individual donors who have raised so much for, or given so generously to, one Israeli cause or another, have not been called upon to publicize the testing or finance it for families in need of help. Like the two wise men in the fairy tale that could just as easily be true, Dr. Rimoin and his colleagues at Cedars-Sinai are standing at the gates of our ancient kingdom, ready to break the spell and free us of the sorcerer’s curse.

A hundred years from now, if someone were to write the story of Iranian Jews in Diaspora, the existence of such gene mutations would no longer seem fictional. Nor would the ability of science to eradicate them as it did Tay-Sachs among Ashkenazi Jews. What would seem unreal is if we fail to seize this chance to save ourselves and our children from a fate that, for all its mythical veneer, is bitterly and irreparably real.

For more information on the Persian Jewish Genetic Screening Program, call (310) 423-4461, or visit www.csmc.edu/medgenetics.

Gina Nahai is professor of creative writing at USC. She can be reached at ginabnahai.com.

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